[Anesthesia for living donor renal transplantation in a patient with Goodpasture's syndrome with a history of repeated alveolar hemorrhage].

A 20-year-old woman was scheduled for living renal transplantation from her mother. She was diagnosed with Goodpasture's syndrome at 8 years of age and had a previous history of alveolar hemorrhage 5 times. She developed renal failure, hypertension and required hemodialysis. She had no history of surgery. Blood test was not remarkable except anemia and elevated blood urea nitrogen and serum creatinine levels. General anesthesia was induced and maintained with fentanyl, remifentanil and propofol. After tracheal intubation, the lungs were mechanically ventilated with a pressure control mode and the peak airway pressure was initially adjusted to 9 cmH2O for maintaining airway pressure at a low level to prevent alveolar injury, which was increased to 12 cmH2O at the end of surgery for maintaining a tidal volume of approximately 250 ml. No hemorrhage was detected in the airway during anesthesia, blood gas data were within the normal range, and the tracheal tube was removed after surgery in the operating room. Postoperative course was uneventful. Antihypertensive agents were discontinued and she was successfully weaned from hemodialysis.

Case Report: Bilateral Lung Transplantation for Rapidly Progressive Undifferentiated Interstitial Lung Disease-A Cautionary Tale.

Interstitial lung disease is fast becoming one of the most common indications for lung transplantation (LTx); however, LTx for Goodpasture's syndrome with pulmonary involvement has not been previously described in the literature. In this report, we outline the case of a young male with undifferentiated rapidly progressive interstitial lung disease who ultimately received a bilateral sequential LTx after deterioration requiring extracorporeal membrane oxygenation. The original disease soon recurred in the graft, and unfortunately, the patient did not survive. The diagnosis of Goodpasture's syndrome was made postmortem and was not clearly evident on examination of the native explanted tissue, nor was there an elevated titer of antiglomerular basement membrane antibodies during his initial work-up. We hypothesize that the donor and recipient's HLA profile made him more susceptible to aggressive disease. In hindsight, active Goodpasture's disease would have been a contraindication to proceed to transplantation. This case is a cautionary reminder of the high stakes of performing LTx without a certain diagnosis.

[Goodpasture's syndrome with neurologic involvement and negative ANCA]. / Síndrome de Goodpasture asociado con vasculitiscerebral ANCA negativa.

Goodpasture's syndrome is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis (RPGN) and alveolar hemorrhage in the presence of antiglomerular basement membrane (anti-GBM) antibodies. Central nervous system involvement is highly unusual in the absence of anti-neutrophil cytoplasmic antibodies. We report the case of a 20-year-old man with RPGN accompanied by bloody sputum, tonic-clonic seizure and high titers of anti-GBM antibody. After treatment with immunosuppressants and plasmapheresis, the patient showed reduced anti-GBM antibody titers and improved neurologic and respiratory symptoms, but renal failure persisted, requiring hemodialysis. Twenty months later, with the disease in remission, he underwent deceased-donor renal transplantation.

[Benefit of Howell-Jolly bodies detection: finding of an acquired hyposplenism in a patient with Goodpasture syndrome]. / Intérêt de la détection des corps de Howell-Jolly : hyposplénisme acquis dans le cadre d'un syndrome de Goodpasture.

Howell-Jolly bodies are intraerythrocytic inclusions corresponding to a small portion of chromatin. Red blood cells that contain these nuclear remnants are removed from the circulation by the spleen. In most cases, presence of Howell-Jolly bodies on a blood smear is the result of functional asplenia and splenectomy. Observation. We report incidental finding of numerous Howell-Jolly bodies in a patient followed by the nephrology department. This microscopic observation of blood smear led to a diagnostic imaging and to the evidence of a reduced spleen, possibly favoured by a history of Goodpasture syndrome in this renal transplant patient without splenectomy. Vaccination and antibioprophylaxy were proposed to prevent infectious risk linked to this splenic hypoplasia. Conclusion. Seeking of Howell-Jolly bodies could be made in every condition associated with a risk of splenic hypoplasia to prevent infectious risk.

A rare fatal complication of bleeding immediately following polytetrafluoroethylene arteriovenous grafting in a lady with Goodpasture syndrome.

We report a 28-year-old female with no history of allergies and recent onset of Goodpasture syndrome who developed life-threatening bleeding immediately after placement of a polytetrafluoroethylene (PTFE) graft as an access for hemodialysis in the left upper limb by an experienced vascular surgeon. In spite of transfusing fresh frozen plasma, packed cells and cryoprecipitate, her prothrombin time (PT), activated partial thromboplastin time and international normalized ratio became progressively worse which were normal at the beginning of the surgery. She had profound hypotension and succumbed within 8 hours. We suspect a rare phenomenon of the interaction of her blood with the PTFE graft causing activation of bleeding and coagulation factors leading to disseminated intravascular coagulation (DIC).

Plasmapheresis and immunosuppressive agents in antibasement membrane antibody-induced Goodpasture's syndrome.

Four patients with rapidly progressive glomerulonephritis and pulmonary hemorrhage (Goodpasture's syndrome) induced by circulating anti-glomerular basement membrane (GBM) antibodies were treated with immunosuppressive agents and varying amounts of plasma exchange. All four patients showed progressive decreases in circulating anti-GBM antibody during therapy. Two patients with established renal failure before therapy showed no improvement in renal function but had a remission from pulmonary disease. In two other patients, renal failure developed early in the course of therapy and required maintenance hemodialysis. Later, their renal function improved coincident with a decrease in circulating anti-GBM antibody. Aggressive measures to reduce the levels of circulating anti-GBM antibody may have a salutory effect on the clinical course of the disease, particularly when undertaken early.

Recurrence of anti-GBM antibody disease twelve years after transplantation associated with de novo IgA nephropathy.

A patient developed recurrent anti-glomerular basement membrane (GBM) antibody (ab) disease after twelve years of an uneventful posttransplant course, clinically accompanied by rapidly rising creatinine. He additionally exhibited coexisting IgA nephropathy at the time of the reappearing anti-GBM disease. Both linear IgG and mesangial IgA were detected by immunofluorescence, and electron microscopy demonstrated mesangial immune complex deposits. To our knowledge, the association of anti-GBM ab disease and IgA nephropathy has not been reported previously.

A case of resistant Goodpasture's syndrome and staghorn calculus--treatment with bilateral nephrectomy.

We report the case of a 49-year-old female with severe Goodpasture's syndrome, staghorn calculus, and subacute thyroiditis. Despite the use of a combined therapy with corticosteroid, cyclophosphamide, azathioprine and plasma exchange, we were unable to suppress the disease activity or normalize the anti-glomerular basement membrane (GBM) antibody levels. Disease remission with a parallel reduction in anti-GBM antibody titer was only achieved after sequential bilateral nephrectomy.

Outcome of renal transplantation in patients with pauci-immune small vessel vasculitis or anti-GBM disease.

AIM: Pauci-immune small vessel vasculitis (SVV) and anti-GBM disease are the most common causes of rapidly progressive glomerulonephritis (RPGN) and they frequently lead to end-stage renal disease. For renal replacement therapy, renal transplantation is the preferred treatment option. However, in patients with glomerular diseases, the outcome of renal transplantation can be adversely affected by recurrence of the original disease. The information in the medical literature on the outcome of renal transplantation in patients with RPGN is limited because most data are derived from case studies and from studies involving a small number of patients. METHODS: We studied the outcome of renal transplantation in patients with pauciimmune SVV or anti-GBM disease, transplanted in our center between 1968 and 2000. Patient and graft survival were compared with a matched control group from our hospital. We specifically looked for any evidence of recurrent disease. RESULTS: Included in the study were 43 patients (31 male, 12 female) with a mean age (+/- SD) of 48 +/- 15 years at transplantation. Patients were diagnosed as Wegener's granulomatosis (n = 8), microscopic polyangiitis (n = 7), renal limited vasculitis (n = 18) and anti-GBM disease (n = 10). The average follow-up was 62 +/- 57 months. No graft was lost due to recurrence of the underlying disease. One patient with Wegener's granulomatosis had a relapse with only extrarenal manifestations 5 months after transplantation. Patient and graft survival at 5 years after transplantation were 77% and 60%. Survival rates were not significantly different from a matched control group of renal transplant patients with other underlying diseases, 79% and 56%, respectively. Patients with pauci-immune SVV or anti-GBM disease developed significantly more malignancies than the control group (p = 0.02). CONCLUSIONS: Recurrence of pauci-immune SVV and anti-GBM disease after transplantation is rare. Renal transplantation can be successfully performed in patients with pauciimmune vasculitis or anti-GBM disease. Physicians should be aware of the greater risk of developing malignancies, especially skin cancer.

Role of bilateral nephrectomy in the treatment of renal disease and hypertension.

The case summaries of 13 patients who were bilaterally nephrectomized for treatment of various renal diseases and hypertension are described. The present concept concerning the role of bilateral nephrectomy in the treatment of renal disease and hypertension is discussed.

[Cadaveric renal transplantation for Goodpasture's syndrome: a case report].

A 19-year-old man with a history of histologically-proven Goodpasture's syndrome (hemoptysis, rapidly progressive glomerulonephritis, and positive anti-glomerular basement membrane (anti-GBM) antibody) was maintained on hemodialysis for 21 months. After steroid pulse therapy and plasmapheresis, his anti-GBM antibody disappeared. His stable condition on dialysis and a session of plasmapheresis prior to surgery allowed him to undergo cadaveric renal transplantation from a 34-year-old man. The blood type was identical (group A and Rh (+)), and there was 1 and 0 mismatch of HLA class 1 and 2, respectively. The initial immunosuppressants included cyclosporine, methylprednisolone, mizoribine, azathioprine, and anti-lymphocyte globulin (ALG). The transplanted kidney regained function on day 6 and he was free from hemodialysis. Circulating anti-GBM antibody was negative after surgery. The graft has functioned well for almost 4 years after transplantation without any episodes of renal or pulmonary complications. To the best of our knowledge, this is the first report of renal transplantation for Goodpasture's syndrome in the Japanese literature.

Kidney transplant after preexisting posterior reversible encephalopathy syndrome induced by Goodpasture's syndrome.

Posterior reversible encephalopathy syndrome is characterized by varying neurologic symptoms associated with brain vasogenic edema. Posterior reversible encephalopathy syndrome can be associated with severe hypertension (eg, in eclampsia or HELLP syndrome), but it also has been observed without hypertension and in several clinical conditions including infections and autoimmune disorders. The literature offers several reports of posterior reversible encephalopathy syndrome detected or induced after bone-marrow and solid-organ transplant, or induction by immunosuppression. We describe what is, to the best of our knowledge, the first case of man who successfully underwent a kidney transplant with preexisting posterior reversible encephalopathy syndrome induced by Goodpasture's syndrome.

Treatment of severe psoriasis with etanercept in a pancreas-kidney transplant recipient.

Severe psoriasis is a rare condition under immunosuppressive therapy. We describe a 42-years-old man with psoriasis since the age of 22 years. The patient underwent a combined pancreas-kidney transplantation at the age of 32 because of Goodpasture syndrome with renal and pulmonary involvments and type 1 diabetes mellitus. Seven years later a pancreas retransplantation was performed due to nonfunction of the original pancreas allograft. Despite intensive systemic immunosuppression, consisting of prednisone, tacrolimus, and mycofenolate mofetil, and topical treatment with dithranol and steroids, there was significant worsening of psoriasis. In October 2009 we initiated therapy with etanercept (25 mg s.c.) twice weekly under close clinical and laboratory monitoring. Improvement was rapid with a decrease in Psoriasis Area and Severity Index (PASI) from 25.2 to <5 during the first months of treatment without a severe infection or other adverse reaction. Graft functions were not affected by the treatment. The patient remains till now on the same regimen and is almost free of skin manifestations. To our knowledge so far only 2 cases of etanercept therapy in psoriasis have been reported in liver transplant recipients. In both cases the treatment was well tolerated and effective. Psoriasis therapy in organ transplant recipients represents a major challenge. Biological agents such as etanercept may provide an effective option for refractory cases.

[Kidney allograft: a target for systemic disease]. / Le rein transplanté : une cible des maladies systémiques.

Recurrence of disease after transplantation is frequent and represents the third cause of allograft loss. Recurrence of lupus nephritis after transplantation is rare. Kidney transplantation in patients with antiphospholipid syndrome or lupus anticoagulant is challenging due to the high risk of immediate post-transplant thrombosis and bleeding risk associated to the subsequent anticoagulation. Moreover, vascular changes associated to the presence of antiphospholipid antibodies negatively impact allograft rate survival. Recurrence of pauci immune glomerulonephritis or Goodpasture syndrome is exceptional.